Cilioretinal Artery Occlusion Research Articles

A Cilioretinal Artery Occlusion (CLRAO) Associated with Optic Disc Edema after Viral Vector SARS-CoV-2 Vaccination: Case Report

A cilioretinal artery occlusion (CLRAO) associated with optic disc edema was diagnosed in a patient seven days after the second dose of viral vector SARS-CoV-2 vaccination. The patient developed sudden blurred vision in the inferior hemifield of the right eye. His fundus examination showed retina edema at the superotemporal quadrant within vascular arcades of the right eye. Fundus fluorescein angiography (FFA), optical coherence tomography (OCT), and optical coherent tomography angiography (OCTA) were used to confirm the diagnosis. Keywords: COVID-19; SARS-Cov2; Cilioretinal artery occlusion; Disc edema; Vaccine

Both Central Retinal Venous Occlusion and Cilioretinal Artery Occlusion Secondary to Hyperhomocysteinemia. A Case Report

A 50 YO female patient with no medical history presented to the ophthalmologic emergency department for an acute decreasing of visual acuity in the left eye without any redness nor ocular pain. Ophthalmic examination revealed a best corrected VA of ‘finger counting’ in the LE. The LE slit lamp examination was normal. Fundus evaluation showed a retinal white edema in the cilioretinal artery territory associated with pre retinal hemorrhage. The foveola was spared (Figure 1).

Idiopathic combined retinal vessels occlusion in a pregnant woman: a case report

BackgroundOcular vascular occlusion is an extremely rare event, especially in the young population. This diagnosis is always associated with active systemic diseases in young adults and needs thrombophilia workup. Nevertheless, we present the case of a pregnant woman suffering from idiopathic combined central retinal vein and cilioretinal artery occlusion.Case presentationA 36-year-old pregnant Iranian woman at the 36th week of her second pregnancy complained of subacute unilateral painless decreased vision of her left eye. She had experienced a transient vision loss that lasted several minutes, but attacks gradually became more frequent and finally persistent over a several-day period. Finally combined central retinal vein and cilioretinal artery occlusion was established. Her visual acuity improved to 4/10 in a month without any ocular intervention except for a short duration of prophylactic dose enoxaparin, and the acuity reached 8/10 without any complications in the third month follow-up visit. At 1 year follow-up, the visual acuity had not changed and no macular edema was seen.ConclusionsOcular vascular occlusion is extremely rare among young adults, and even rarer among pregnant women. According to this, any suspected retinal vascular event in this population should raise suspicion for underlying diseases such as hypertension, diabetes, autoimmune diseases, migraine, preeclampsia syndrome, and thrombophilia. However, as seen in this presented case, idiopathic ocular vascular occlusion events can occur also.

P298 Ocular manifestations in a giant cell arteritis cohort

Abstract Background/Aims The ophthalmic features of giant cell arteritis involve a spectrum of severity, from transient symptoms to bilateral visual loss due to anterior ischaemic optic neuropathy or, less commonly, central retinal or cilioretinal artery occlusion. Data of reported ocular involvement are varied, with a range of 10-70%. The variation may be related to the specialty of the unit reporting the data. We run an interdisciplinary service in a tertiary academic unit and report a complete picture of the frequency and nature of ocular involvement in GCA. Methods The records of 350 consecutive patients, diagnosed objectively on the basis of biopsy or imaging, were reviewed. In our centre, the hospital pathway mandates all individuals with suspected GCA to have a formal ophthalmology assessment including visual acuity, pupil exam and full dilated fundus assessment. We recorded the common signs and symptoms using a structured form which included ophthalmologic symptoms and examination findings, along with symptoms commonly associated with GCA: scalp tenderness, headache, jaw claudication, shoulder girdle stiffness, anorexia, night sweats, weight loss, and fever. Results From January 2012 to September 2021, 350 individuals were diagnosed with GCA by biopsy, ultrasonography or positron emission tomography. The mean age was 74 ± 7.7 years. 237 (68%) of patients were females. 99 (28%) presenting with GCA had visual symptoms and or signs. 34 had mono-ocular and two had binocular loss of vision. A summary of the key visual symptoms and signs are shown in Table 1. Only five patients with visual symptoms did not have any symptoms commonly associated with GCA. Conclusion We report the frequency of visual involvement in one of the largest cohorts of individuals with GCA. 28% have ocular symptoms. Partial or total monocular field loss occurred in 12% of cases. 1.4% of patients may be presenting with visual symptoms as the only sign of GCA. Rarely, permanent visual loss may occur without any other manifestation of GCA. Disclosure S. Mansfield Smith: None. M. Al-Hashimi: None. G. Ducker: None. C. Jones: None. C. Mukhtyar: None.

Retinal vascular occlusion in pregnancy: three case reports and a\xa0review of the literature

BackgroundRetinal arterial occlusive events in young patients are rare. However, because of physiological multifactorial adaptations during pregnancy, retinal vascular occlusive disease may occur spontaneously. In addition, a patent foramen ovale is a risk factor for an ischemic thromboembolic event. Since fluorescein angiography, a central tool in the evaluation of these occlusions, should be avoided during pregnancy, optical coherence tomography angiography, a novel technique, offers a good opportunity for visualizing vascular perfusion of retinal tissue.Case presentationHere we present a case series of three patients (Caucasian, nonsmoker) who visited our clinic owing to acute visual impairment and central scotoma. Using regular optical coherence tomography and optical coherence tomography angiography, retinal vascular occlusions were detected, thus initiating the evaluation of systemic risk factors. We report two patients (30 and 32 years old) who developed cilioretinal artery occlusion but whose etiology differed: one was of thromboembolic origin associated with patent foramen ovale, while the other was caused by hemodynamic blockade secondary to central retinal vein occlusion. In both cases, optical coherence tomography angiography revealed reperfusion of the cilioretinal artery occlusion. However, transient ischemia led to retinal atrophy after a few weeks. In the third patient (32 years old), 8 weeks after onset of scotoma, optical coherence tomography angiography revealed atrophy of the middle layers and impaired perfusion in the deep capillary plexus, and thus a paracentral acute middle maculopathy was diagnosed. All patients regained normal visual acuity and had otherwise uncomplicated pregnancies, and laboratory blood tests did not reveal any defects or alterations.ConclusionsAs shown here, optical coherence tomography angiography enables risk-free imaging of retinal vessel perfusion during pregnancy. Together with regular optical coherence tomography, it allows one to predict functional outcome according to the existing retinal occlusion-related atrophy.

Cilioretinal Artery Occlusion Following Diagnostic Lumbar Puncture in a Patient of Idiopathic Intracranial Hypertension.

Cilioretinal Artery Occlusion Following Diagnostic Lumbar Puncture in a Patient of Idiopathic Intracranial Hypertension.

Heterozygous factor V Leiden mutation manifesting with combined central retinal vein occlusion, cilioretinal artery occlusion, branch retinal artery occlusion, and anterior ischaemic optic neuropathy: a case report

BackgroundOur purpose was to describe a patient who developed combined central retinal vein occlusion (CRVO), cilioretinal artery occlusion, branch retinal artery occlusion (BRAO), and anterior ischaemic optic neuropathy (AION) followed by CRVO in the second eye because of the heterozygous factor V Leiden (FVL) mutation.Case presentationA 39-year-old female with a history of recurrent pregnancy losses presented with acute blurred vision in the right eye (RE), with visual acuity limited to counting fingers. She was diagnosed with combined impending CRVO, cilioretinal artery occlusion, BRAO, and AION. The results of thrombophilia testing, not including the FVL mutation, were negative. Retinal atrophy with vascular attenuation and optic disc pallor developed after resolution of acute retinal findings. Nine months after initial presentation, the patient developed an impending CRVO in the left eye (LE), with a secondary progression to a complete CRVO causing a decrease in best corrected visual acuity (BCVA) to 20/40. The patient was determined to be heterozygous for the FVL mutation. She subsequently was treated with acenocoumarol. At the last follow-up visit, the BCVA was 20/400 in the RE and 20/20 in the LE, and there was a complete resolution of the acute CRVO findings in the LE.ConclusionOur case shows that the heterozygous FVL mutation may manifest with combined retinal vascular occlusion involving multiple sites in both eyes. Early recognition of such an inherited thrombophilic disorder is important because it implies the need for long-term anticoagulative therapy to reduce the patient’s risk of recurrent, sight-threatening and life-threatening thrombotic events.

Actue Isolated Cilioretinal Artery Occlusion Secondary to Percutaneous Coronary Intervention: A Case Report

Actue Isolated Cilioretinal Artery Occlusion Secondary to Percutaneous Coronary Intervention: A Case Report

COVID-19 İlişkili Silioretinal Arter Tıkanıklığı

A 49-years old woman patient presented with acute central loss of vision, with the diagnosis of cilioretinal artery occlusion was hospitalized. She had a history of close contact with a coronavirus disease-2019 (COVID-19) patient and her polymerase chain reaction test was positive. Topical and systemic treatments were used to reduce intraocular pressure. In addition, anticoagulant treatment was applied. After excluding other prothromboembolic etiological factors, the patient was diagnosed with cilioretinal occlusion with associated COVID19. Thromboembolic complications associated with COVID-19 have a high incidence and may affect the retinal vascular structure. We described a case of cilioretinal artery occlusion associated with COVID19 infection. We might suggest a pharmatherapeutical prophylaxis for COVID-19 associated ocular vascular thromboembolic complications not only in symptomatic patients but also in asymptomatic patients. We hope that this case report will contribute to a different perspective on thrombophilia and related conditions in COVID-19.

Surgical mobilization of an arterial embolus in cilioretinal artery occlusion.

We describe an effective surgical approach for the management of cilioretinal artery occlusion. A 23-G pars plana vitrectomy assisted with two soft tip cannulas was performed. One cannula pressed the cilioretinal artery branch directed toward the macula, distal to the location of the embolus, whereas the other cannula was used to gently swipe over the cilioretinal artery proximal to the occlusion. Anatomical and functional outcomes were evaluated by fundus examination, fluorescein angiography, Goldmann visual field, and best-corrected visual acuity (BCVA). It was possible to mobilize the embolus by mechanical displacement with 23-G soft-tip cannulas and disintegrate it, preventing the passage toward the branch directed to the macula. Restoration of retinal circulation was confirmed by fluorescein angiogram. The patient recovered his previous documented BCVA and visual field. The described technique can be considered as a new possibility for achieving a solution to cilioretinal artery occlusion or any other retinal artery occlusion.

O31 Giant cell arteritis - a rare ocular presentation

O31 Giant cell arteritis - a rare ocular presentation

Outcomes and lessons learned from two decades’ experience with glaucoma drainage device implantation for refractory Sturge Weber–associated childhood glaucoma

To evaluate glaucoma drainage device (GDD) implantation for refractory pediatric glaucoma associated with Sturge-Weber syndrome (SWS). The medical records of consecutive children with SWS-associated glaucoma at a single center who were treated by a single surgeon using GDDs over 20years were reviewed retrospectively. The main outcome measure was GDD treatment success, defined as absence of any of the following indications of treatment failure: (1) intraocular pressure (IOP) of >21mm Hg on two consecutive visits despite maximal medical therapy, (2) additional IOP-lowering surgery, and (3) sight-threatening complications. A total of 22 eyes of 22 children were included. The median age at glaucoma diagnosis was 0.73years (range, 0.06-13.2), and the median age at GDD surgery was 4.8years (range, 0.6-13.3). Most eyes (14 [68%]) had prior glaucoma surgery. Mean follow-up was 2.8±1.5years. Success (95% confidence interval) by Kaplan-Meier analysis for GDD surgery at 1, 3, and 5years was 91% (68-98), 75% (50-89), and 52% (24-73), respectively. Failure occurred in 8 eyes (36%). Complications were common, occurring in 50% of eyes, with 23% of eyes having more than one complication. Severe vision-threatening complications (n=3) included one case each of possible infection requiring GDD removal, persistent hypotony, and cilioretinal artery occlusion. GDDs are an effective treatment for SWS-associated glaucoma but have a high rate of complications. We report several severe complications that prompted surgical modifications for increased safety when implanting GDDs in eyes with SWS-associated glaucoma.

Combined Central Retinal Vein and Cilioretinal Artery Occlusion in Pregnancy treated with Intravitreal Triamcinolone Acetonide

Combined Central Retinal Vein and Cilioretinal Artery Occlusion in Pregnancy treated with Intravitreal Triamcinolone Acetonide

English

BACKGROUND Central retinal artery occlusion (CRAO) was first described by Van Graefe in 1859 as an embolic event to the central retinal artery in a patient with endocarditis. CRAO has various causes, but patients typically present with sudden, severe, and painless loss of vision. Retinal arterial occlusions are a cause for profound visual loss in the population. Carotid atherosclerosis is common in elderly people. Dyslipidaemia, hypertension, and diabetes mellitus are factors which accelerate the development of carotid atheromatous plaques. Embolism from the carotid bifurcation is the most common cause of retinal artery occlusions. In retinal arterial occlusion carotid arterial occlusion is usually assessed using radiological techniques. The purpose of this study was to evaluate carotid atherosclerotic disease in patients with arterial occlusions in the eye and determine the relation between arterial occlusions in the eye & carotid artery occlusive disease. METHODS This retrospective study included patients aged thirty and above, who had come with symptoms suggestive of arterial occlusions in the eye and carotid doppler was done. The inclusion criteria included patients diagnosed with the following conditions CRAO, branch retinal artery occlusion (BRAO), ophthalmic artery occlusion, anterior ischaemic optic neuropathy (AION) and cilioretinal artery occlusion. Patients usually present with sudden loss of vision in one eye. After taking a detailed history, all patients were subjected to a thorough ocular examination. Patient’s vision is assessed using Snellen’s visual acuity chart, pupillary assessment done, and fundus examination to look for retinal arterial occlusion is also done. Fundus imaging is also done. Carotid doppler was done to rule out carotid artery occlusive disease. RESULTS Patients presenting with retinal arterial occlusion should be investigated thoroughly for both systemic and local causes of CRAO. The risk of developing arterial occlusions were 1.7 - 9.15 times more in patients with carotid artery occlusion than in patients with normal carotids. Arterial occlusion was more found in patients with 70 % occlusion of the carotid artery. CONCLUSIONS There was a strong association between retinal arterial occlusions and carotid artery occlusion. KEYWORDS Central Retinal Artery Occlusion (CRAO), Carotid Artery Occlusive Disease, Carotid Doppler

Vision Loss following Small Incision Cataract Surgery: A Case Report

Introduction: Retro-bulbar block is still safe and widely employed anesthetic technique in large volume routine cataract surgery. Nevertheless, the procedure is not free of complication. Case report: A 48 years old man who had small incision cataract surgery of left eye under retro-bulbar block, had vision of hand movement close to face on his first postoperative day. He was given intracameral cefuroxime and subconjunctival gentamycin at the end of the surgery. Fundoscopy of the operated eye showed marked retinal whitening with cherry-red spot in the posterior pole suggestive of cilio-retinal artery occlusion. Optical coherence tomography and optical coherence tomography angiography were done to support and confirm the diagnosis. Conclusion: Retinal artery occlusion secondary to retro-bulbar block or drug induced toxicity following routine cataract surgery is unusual and sometimes dreadful vision threatening complication can occur.

Cilioretinal Artery Occlusion after Endoscopic Sinus Surgery: A Rare and Hardly Remembered Complication

The proximity between nasal and sinus structures and the orbit causes ocular complications to occur with some frequency in endoscopic sinus surgery (ESS). Among them, vascular ones are rare. Only 3 cases of post-ESS retinal artery obstruction have been published to date. Despite the low frequency, arterial occlusions can cause irreversible damage to vision. In the present report, we present a case of a patient who evolved with occlusion of the cilioretinal artery, leading to scotoma formation in the left eye, after ESS. The present report highlights the importance of the otolaryngologist's knowledge of this complication, which, although rare, has high morbidity.

Combined cilioretinal artery and vein occlusion and glaucoma: genetics in two siblings

PurposeHere we present two siblings with combined cilioretinal artery and vein occlusion (CLARVO), with genetic confirmation of glaucoma as the underlying etiology and discovering of two novel variants of CARD10 and OLFM2.MethodsAncillary tests were performed to rule out other etiologies, and genetic tests using next generation sequencing (NGS) were performed in the two siblings. An extensive literature research was performed of similar cases of CLARVO and glaucoma, as well as the genetics (NCBI Genes Database) found in our patients.ResultsIn April 2016, a 39‐years‐old man and a 45‐year‐old woman came to the Emergency Room referring visual loss on their right eye, also both with an onset of the symptoms differing only two weeks. They denied any abnormalities, recent travels or drugs abuse. On the examination, both presented CLARVO in the right eye, a more severe condition in the female patient along with lower visual acuity. Both patients showed a pigmentary dispersion glaucoma with a higher cup‐to‐disc ratio in their right eye. IOP was controlled with medical treatment, and in the female peripheral iridotomies and selective trabeculoplasty were also needed. A genetic analysis with NGS showed variants of CARD10 (pThr436Met) in both siblings, and in OLFM2 (p.ARg460=) in the female. These genes have been related to a higher risk of increased IOP (CARD10) and normal development of the structures of the eye (OLFM2). Also, OLFM2 has been recently linked to vascular smooth muscle cells regulation.ConclusionsCLARVO is a rare entity. As in other vascular retinal diseases, like in retinal vein occlusion, even in younger patients, classic predisposing factors like glaucoma should be considered. When available, genetic tests should be performed, especially in presumed familiar association. The combination of the genetic variants in our patients could be related to both a higher predisposition of high‐pressure glaucoma, along with an increased susceptibility of the retinal vasculature; however, further research is needed to confirm this association between CLARVO and glaucoma.

Commentary: COVID-19 disease is more than just a respiratory infection

The coronavirus disease (COVID-19), caused by the novel, highly contagious severe acute respiratory syndrome coronavirus 2, has quickly become a global health emergency. It started with an outbreak of pneumonia causing fever, cough, and shortness of breath. However, the virus has been noted to involve multiple organs and systems. The global research community has made an impressive effort in compiling the various manifestations of the disease in a short time.[1] The infected individuals are predisposed to arterial and venous thromboembolic complications like deep vein thrombosis, pulmonary embolism, myocardial infarction, and stroke. The proposed mechanisms responsible for these thromboembolic events include a transient hypercoagulable state due to systemic inflammation and cytokine storm, postinfectious immune-mediated responses, and direct viral-induced endothelitis.[2] The incidence of thrombotic complications in COVID-19 patients admitted to intensive care unit ranges between 16 to 49%.[1] Acute cardiovascular accident has been reported in 0.5–6.0% of COVID-19 patients.[3] Acharya et al.[4] first reported a central retinal artery occlusion (CRAO) in a case of severe COVID-19 disease. However, recent evidence suggests that arterial thromboembolism can occur in patients with mild disease also. Currently, the incidence is unknown due to possible underreporting of symptoms. Turedi et al. first reported CRAO within a case of moderate COVID-19 disease.[5] Early anticoagulant treatment has been reported to improve the clinical outcome of COVID-19 patients and reduce the risk of thromboembolic events.[1] Interestingly, all the patients that have been reported to develop RAO secondary to COVID-19 were already being treated with anticoagulant therapy. The occurrence of RAO in COVID-19 patients is limited to only case reports. Further studies should be planned to evaluate out the incidence of vascular occlusions in patients suffering and recovered from COVID-19. Such studies can give us insight related to the association of the ocular diseases with the stage and severity of the COVID-19 infection. Our knowledge regarding the pathophysiology and treatment of this disease is still in its preliminary stage. The fast-paced ongoing research will hopefully provide better algorithms for management of this disease and prevention of its complications. Physicians treating COVID-19 patients must be sensitized to seek an urgent retina consultation for patients complaining of symptoms like blurry vision, sudden painless visual loss, or scotomas. We congratulate the authors for reporting the first case of an isolated cilioretinal artery occlusion (CLRAO) associated with paracentral acute middle maculopathy (PAMM) in a patient with moderate COVID-19 disease and no other predisposing diseases. PAMM is reported to be present in 100% of isolated CLRAO due to hypoperfusion of the involved cilioretinal artery.[6] The authors started treating the patient 48 h after the episode and gave 20 sessions (40 h) of hyperbaric oxygen (HBO), which led to resolution of retinal edema after 3 weeks. HBO involves the inhalation of 100% oxygen at pressures greater than 1 atm. The inspired oxygen partial pressure is nearly 203 kPa, which is almost 10 times that of breathing air at normal atmospheric pressure. It is postulated that oxygen at higher pressures diffuses from the choroidal circulation to reach the ischaemic retina and increase the oxygen delivery to the ischemic tissue until spontaneous or assisted reperfusion occurs.[7] However, isolated CLRAO is associated with good prognosis and does not usually require treatment. Retinal edema usually resolves spontaneously within few weeks. The central field defect improves in nearly half of the eyes and remains stable in the other half.[8] HBO is usually reserved for patients presenting with poor vision due to CRAO and BRAO. Also, the maximum benefit of the treatment is expected in case it is started within 6–8 h of the occlusion.[7] The risk-benefit for the application of multiple sessions to gain <1 line of vision during the COVID-19 pandemic should be carefully calculated. Role of corticosteroids in COVID-19 has been evaluated by several studies. The RECOVERY trial was a prospective study where 2104 patients were randomized to dexamethasone 6 mg per day (oral or intravenous) for 10 days and compared to the 4321 patients receiving usual care. They concluded that in patients hospitalized with COVID-19, dexamethasone reduced the 28-day mortality among those receiving invasive mechanical ventilation or oxygen at randomization, but not among patients with mild to moderate COVID-19 disease.[9]

Hyperbaric oxygen treatment of central retinal vein occlusion with cilioretinal artery occlusion secondary to hormonal treatment: Case report and review

(Khallouli A, Khelifi K, Saidane R, Choura R, Maalej A, Ben Sassi R. Hyperbaric oxygen treatment of central retinal vein occlusion with cilioretinal artery occlusion secondary to hormonal treatment: Case report and review. Diving and Hyperbaric Medicine. 2020 December 20;50(4):431–436. doi: 10.28920/dhm50.4.431-436. PMID: 33325028.) Introduction: This report describes a case of central retinal vein occlusion (CRVO) and cilioretinal artery occlusion (CLRAO) after hormonal treatment for induction of ovulation that was successfully treated with hyperbaric oxygen. Case report: A 48 year-old woman was admitted to our department for sudden blurred vision in her left eye. The patient had a history of 3-months hormonal treatment for induction of ovulation. The best corrected visual acuity was 7/10 (20/32) in the left eye and 10/10 (20/20) in the right eye. Fundus examination of the left eye revealed flame-shaped haemorrhages, whitening of the retina along the distribution of cilioretinal artery and tortuous retinal veins. Fluorescein angiography confirmed the combination of a non-ischaemic CRVO with CLRAO. The patient was treated with a 2 h session of hyperbaric oxygen at 253 kPa (2.5 atmospheres absolute) once daily for a total of 30 sessions. Best corrected visual acuity improved to 10/10 (20/20) in the left eye. Conclusions: CRVO and CLRAO are both occlusive disorders. HBOT is a safe low-cost treatment modality that can be beneficial in some ocular pathologies. It can maintain oxygenation of the retina through the choroidal blood supply, decrease oedema and preserve compromised tissue adjacent to the ischaemic area.

Bilateral cilioretinal artery occlusions in giant cell arteritis

An 82-year-old man presented with painless sequential vision loss of his right eye (OD). Ten days earlier, he presented to another hospital for left eye (OS) vision loss. His vision was Light perception only OD and No light perception OS. Fundoscopy demonstrated bilateral cilioretinal artery occlusions with chalky white optic disc swelling OS (Fig. A, B). Although he denied symptoms of giant cell arteritis (GCA) and had normal inflammatory markers, his clinical examination was suspicious for GCA, which was confirmed by temporal artery biopsy.